INTERNATIONAL CLASSIFICATION OF DISEASES -

Mortality and Morbidity Statistics

ICD-11 MMS – 09/2020

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CHAPTER 09

Diseases of the visual system

This chapter has 159 four-character categories.

Code range starts with 9A00

This refers to any diseases of the visual system, which includes the eyes and adnexa, the visual
pathways and brain areas, which initiate and control visual perception and visually guided behaviour.

Exclusions:

Certain conditions originating in the perinatal period (Chapter 19)

Certain infectious or parasitic diseases (Chapter 01)

Complications of pregnancy, childbirth and the puerperium (Chapter 18)

Endocrine, nutritional or metabolic diseases (Chapter 05)

Injury, poisoning or certain other consequences of external causes (Chapter 22)

Posterior cortical atrophy (8A21.0)

Coded Elsewhere:

Neoplasms of the eye or ocular adnexa

Reasons for contact with the health care system in relation to eyes or vision

Contusion of eyeball or orbital tissues (NA06.9)

Foreign body in multiple parts of external eye (ND70.2)

Oculocutaneous albinism (EC23.20)

Traumatic injury to eyeball (NA06.8)

Birth injury to eye (KA41)

Late congenital syphilitic oculopathy (1A60.2)

Symptoms, signs or clinical findings of the visual system (MC10-MC2Y)

Structural developmental anomalies of the eye, eyelid or lacrimal apparatus

(LA10-LA1Z)

This chapter contains the following top level blocks:



Disorders of the ocular adnexa or orbit



Disorders of the eyeball - anterior segment



Disorders of the eyeball - posterior segment



Disorders of the eyeball affecting both anterior and posterior segments



Disorders of the visual pathways or centres



Glaucoma or glaucoma suspect



Strabismus or ocular motility disorders



Disorders of refraction or accommodation



Postprocedural disorders of eye or ocular adnexa



Impairment of visual functions

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

Vision impairment



Neoplasms of the eye or ocular adnexa



Reasons for contact with the health care system in relation to eyes or vision

Disorders of the ocular adnexa or orbit (BlockL1‑9A0)

Coded Elsewhere:

Ocular myiasis (1G01.0)

Disorders of eyelid or peri-ocular area (BlockL2‑9A0)

Coded Elsewhere:

Congenital malformations of the eyelid

Seborrhoeic keratosis (2F21.0)

Cysts of eyelid (2F36.4)

Eyelid trauma (NA06.0)

Benign cutaneous neoplasm or cyst of eyelid (2F36.Y)

9A00

Congenital malposition of eyelids
Coded Elsewhere:

Congenital entropion (LA14.02)

Congenital ectropion (LA14.03)

Congenital ptosis (LA14.04)

Hypotelorism (LB71.0)

Hypertelorism (LB71.1)

Epiblepharon (LA14.0Y)

9A00.0

Dystopia canthorum

Coded Elsewhere:

Waardenburg syndrome (EC23.2Y)

9A00.1

Telecanthus

9A00.Y

Other specified congenital malposition of eyelids

9A00.Z

Congenital malposition of eyelids, unspecified

9A01

Infectious disorders of eyelid
Coded Elsewhere:

Trachoma (1C23)

Involvement of eyelid in tuberculosis (1B12.1)

Involvement of eyelid in leprosy (1B20.3)

Verruca vulgaris of eyelid (1E80.Y)

9A01.0

Preseptal cellulitis

9A01.1

Abscess of eyelid

9A01.2

Hordeolum
An acute focal infection usually by Staphylococcus aureus involving the eyelash
follicle and its associated meibomian and Zeis glands. If the principal focus of
infection is the follicle, it presents with a painful boil which discharges pus at the

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eyelid margin (external hordeolum or stye). If the infection is centred on the
meibomian gland (internal hordeolum) then suppuration onto the conjunctival
surface occurs.

9A01.20

Hordeolum externum
An acute focal pyogenic infection of the eyelash follicle commonly known as a stye
and caused predominantly by Staphylococcus aureus. It presents as an acute
painful inflammatory eyelid swelling which subsequently discharges at the eyelid
margin.

9A01.21

Hordeolum internum
A focal acute pyogenic infection, usually by Staphylococcus aureus, of a meibomian
gland, the normal secretion from which into the eyelash follicle is blocked. It
presents as an acute inflammatory swelling which may discharge onto the
conjunctival surface of the eyelid, or rarely anteriorly through the eyelid skin. It may
predispose to formation of a chalazion.

Exclusions:

Chalazion (9A02.0)

9A01.2Z

Hordeolum, unspecified

9A01.3

Infectious blepharitis
A condition of the eyelid, commonly caused by an infection with a bacterial source.
This condition is characterised by pruritus, burning, scratchiness, excessive tearing,
or crusty debris around the eyelashes. This condition may also present with lid
erythema, collarettes, madarosis, trichiasis, or plugged meibomian glands.
Transmission is by direct or indirect contact with an infected individual, endogenous
spread, or through fomites.

Exclusions:

Blepharoconjunctivitis (9A60.4)

Coded Elsewhere:

Herpes simplex infection of eyelid (1F00.11)

Molluscum contagiosum of eyelid (1E76)

Zoster infection of eyelid (1E91.1)

9A01.4

Infestation of eyelid

Coding Note:

Code aslo the casusing condition

Coded Elsewhere:

Parasitic infestation of eyelid in loiasis (1F66.0)

Parasitic infestation of eyelid in leishmaniasis (1F54.Z)

9A01.Y

Other specified infectious disorders of eyelid

9A02

Inflammatory disorders of eyelid
Coded Elsewhere:

Atopic eczema of eyelids (9A06.70)

Seborrhoeic dermatitis of eyelids (9A06.71)

9A02.0

Chalazion
A chalazion is a small cyst on the eyelid caused by blockage of a meibomian gland.

9A02.00

Chalazion externum

9A02.01

Chalazion internum

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9A02.0Y

Other specified chalazion

9A02.0Z

Chalazion, unspecified

9A02.1

Posterior blepharitis
Posterior blepharitis is inflammation of the eyelids secondary to dysfunction of the
meibomian glands. Like anterior blepharitis it is a bilateral chronic condition and
manifested by a broad spectrum of symptoms involving the lids including
inflammation and plugging of the meibomian orifices and production of abnormal
secretion upon pressure over the glands. It may be associated with skin rosacea.

9A02.2

Ligneous conjunctivitis
Ligneous conjunctivitis (LC) is a rare form of chronic conjunctivitis characterised by
the recurrent formation of pseudomembranous lesions most commonly on the
palpebral surfaces. It is most frequently reported as a clinical manifestation of
severe homozygous or compound-heterozygous hypoplasminogenaemia. Most
cases involve infants and children.

9A02.4

Meibomian Gland Dysfunction
This refers to the dysfunction of a special kind of sebaceous gland at the rim of the
eyelids inside the tarsal plate, responsible for the supply of meibum, an oily
substance that prevents evaporation of the eye's tear film. Meibum prevents tear
spillage onto the cheek, trapping tears between the oiled edge and the eyeball, and
makes the closed lids airtight.

9A02.Y

Other specified inflammatory disorders of eyelid

9A03

Acquired malposition of eyelid

9A03.0

Blepharoptosis
Drooping of the upper lid due to deficient development or paralysis of the levator
palpebrae muscle.

9A03.00

Marcus-Gunn syndrome
Marcus-Gunn syndrome is characterised by ptosis associated with maxillopalpebral
synkinesis. The syndrome is generally unilateral and sporadic, but bilateral and
autosomal dominant inherited cases have been reported.

9A03.01

Mechanical ptosis of eyelid

9A03.02

Myogenic ptosis of eyelid
This refers to a contraction initiated by the myocyte cell itself instead of an outside
occurrence or stimulus such as nerve innervation, causing drooping or falling of the
eyelid. The drooping may be worse after being awake longer, when the individual's
muscles are tired.

9A03.03

Paralytic ptosis of eyelid

9A03.0Y

Other specified blepharoptosis

9A03.0Z

Blepharoptosis, unspecified

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9A03.1

Entropion of eyelid

9A03.10

Cicatricial entropion of eyelid

9A03.11

Mechanical entropion of eyelid

9A03.12

Senile entropion of eyelid
This is a senile condition in which the eyelid (usually the lower lid) folds inward. It is
very uncomfortable, as the eyelashes constantly rub against the cornea and irritate
it. Entropion is usually caused by genetic factors and very rarely it may be
congenital when an extra fold of skin grows with the lower eyelid (epiblepharon).
Entropion can also create secondary pain of the eye (leading to self trauma,
scarring of the eyelid, or nerve damage).

9A03.13

Spastic entropion of eyelid
This is a spastic condition in which the eyelid (usually the lower lid) folds inward. It
is very uncomfortable, as the eyelashes constantly rub against the cornea and
irritate it. Entropion is usually caused by genetic factors and very rarely it may be
congenital when an extra fold of skin grows with the lower eyelid (epiblepharon).
Entropion can also create secondary pain of the eye (leading to self trauma,
scarring of the eyelid, or nerve damage).

9A03.1Y

Other specified entropion of eyelid

9A03.1Z

Entropion of eyelid, unspecified

9A03.2

Ectropion of eyelid
The turning outward (eversion) of the edge of the eyelid, resulting in the exposure of
the palpebral conjunctiva.

9A03.20

Cicatricial ectropion of eyelid

9A03.21

Mechanical ectropion of eyelid

9A03.22

Senile ectropion of eyelid

9A03.23

Spastic ectropion of eyelid

9A03.24

Floppy eyelid syndrome
Acquired disorder of unknown origin, manifested by an easily everted floppy upper
eyelid and papillary conjunctivitis of the upper palpebral conjunctiva. It is primarily
associated with obese men and obstructive sleep apnoea. The tarsus of the upper
eyelid becomes softer and looser probably due to mechanical forces and
enzymatical changes. The upper eyelid everts during sleep, resulting in irritation,
papillary conjunctivitis, and conjunctival keratinization. Effective treatment consists
of preventing the upper eyelid from everting while the patient is sleeping.

9A03.2Y

Other specified ectropion of eyelid

9A03.2Z

Ectropion of eyelid, unspecified

9A03.3

Eyelid retraction

9A03.4

Lagophthalmos

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9A03.40

Cicatricial lagophthalmos

9A03.41

Mechanical lagophthalmos

9A03.42

Paralytic lagophthalmos

9A03.4Y

Other specified lagophthalmos

9A03.4Z

Lagophthalmos, unspecified

9A03.5

Dermatochalasis of eyelid

9A03.Y

Other specified acquired malposition of eyelid

9A03.Z

Acquired malposition of eyelid, unspecified

9A04

Acquired disorders of eyelashes
Exclusions:

Distichiasis (LA14.0)

Structural developmental anomalies of eyelids (LA14.0)

9A04.0

Trichiasis without entropion
This refers to abnormally positioned eyelashes that grow back toward the eye,
touching the cornea or conjunctiva. This can be caused by infection, inflammation,
autoimmune conditions, congenital defects, eyelid agenesis and trauma such as
burns or eyelid injury. This diagnosis is without a condition in which the eyelid
(usually the lower lid) folds inward. It is very uncomfortable, as the eyelashes
constantly rub against the cornea and irritate it.

9A04.1

Madarosis of eyelid or periocular area
Partial or complete loss of eyelashes and/or eyebrow hairs. Alopecia areata and
chronic cutaneous lupus erythematosus are well recognised causes. If the
underlying cause is known this should be coded as well.

9A04.Y

Other specified acquired disorders of eyelashes

9A05

Movement disorders of eyelid
Exclusions:

Tic disorders (8A05)

Coded Elsewhere:

Benign essential blepharospasm (8A02.00)

Hemifacial spasm (8B88.2)

Facial tic (8A05.03)

9A05.0

Myokymia of eyelid
Myokymia is used to describe an involuntary eyelid muscle contraction, typically
involving the lower eyelid or less often the upper eyelid. It occurs in normal
individuals and typically starts and disappears spontaneously. However, it can
sometimes last up to three weeks. Since the condition typically resolves itself,
medical professionals do not consider it to be serious or a cause for concern.

Exclusions:

Facial myokymia (8B88.1)

Myokymia (MB47.50)

9A05.1

Eyelid apraxia

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9A05.Y

Other specified movement disorders of eyelid

9A05.Z

Movement disorders of eyelid, unspecified

9A06

Certain specified disorders of eyelid

9A06.0

Involvement of eyelid by dermatosis classified elsewhere
Involvement of eyelid by skin diseases such as psoriasis or lichen planus.

9A06.1

Vitiligo of eyelid or periocular area

9A06.2

Symblepharon, acquired

9A06.3

Traumatic scar of eyelid

9A06.4

Xanthelasma of eyelid
Xanthelasmata are a form of plane xanthoma which manifest as sharply
demarcated yellowish deposits of lipid within the skin of the eyelid. While they are
neither harmful nor painful, these minor growths may be disfiguring and may be the
presenting sign of hypercholesterolaemia. They are common in people of Asian
origin and those from the Mediterranean region.

9A06.5

Tear Trough Deformity

9A06.6

Sunken Sulcus Deformity

9A06.7

Dermatitis or eczema of eyelids
Eczematous blepharitis and contact dermatitis affecting the eyelids.

Coded Elsewhere:

Irritant contact blepharoconjunctivitis (EK02.11)

9A06.70

Atopic eczema of eyelids
Atopic eczema affecting the eyelids. This is a common manifestation of atopic
eczema and can result in a significant impact on normal vision and on well-being.

9A06.71

Seborrhoeic dermatitis of eyelids
Seborrhoeic dermatitis of eyelids (seborrhoeic blepharitis) is common. It is
characterised by redness and scaling on the skin of the eyelids with variable
involvement of the eyelid margins.

Exclusions:

Seborrhoea (ED91.2)

9A06.72

Allergic contact blepharoconjunctivitis
Allergic contact dermatitis affecting the eyelid and conjunctivae.

9A06.7Y

Other specified dermatitis or eczema of eyelids

9A06.7Z

Dermatitis or eczema of eyelids, type unspecified

9A06.8

Blepharochalasis
This is a malposition of the eyelid caused either by involution or by inflammation of
the eyelid. The inflammation is characterised by exacerbations and remissions of
eyelid oedema, which results in a stretching and subsequent atrophy of the eyelid
tissue resulting in redundant folds over the lid margins. It typically affects only the

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upper eyelids, and may be unilateral as well as bilateral.

9A06.Y

Other specified disorders of eyelid

9A0Y

Other specified disorders of eyelid or peri-ocular area

9A0Z

Disorders of eyelid or peri-ocular area, unspecified

Disorders of lacrimal apparatus (BlockL2‑9A1)

Exclusions:

congenital malformations of lacrimal system (LA14.1)

9A10

Disorders of lacrimal gland

9A10.0

Infections of the lacrimal gland

9A10.1

Orbital inflammatory syndrome
This refers to a marginated mass-like enhancing soft tissue involving any area of
the orbit. It is the most common painful orbital mass in the adult population, and is
associated with proptosis, cranial nerve palsy (Tolosa-Hunt syndrome), uveitis, and
retinal detachment.

9A10.2

Benign lymphoepithelial lesion of lacrimal gland
This is a type of benign enlargement of the parotid and/or lacrimal glands. This
pathologic state is sometimes, but not always, associated with Sjögren's syndrome.
This diagnosis of paired almond-shaped glands, one for each eye, that secrete the
aqueous layer of the tear film.

9A10.3

Hyperlacrimation

9A10.4

Underproduction of tears
Underproduction of tears causes keratoconjunctivitis sicca and can be caused by
disorders that interrupt the neural control of lacrimation.

9A10.Y

Other specified disorders of lacrimal gland

9A10.Z

Disorders of lacrimal gland, unspecified

9A11

Disorders of lacrimal drainage system
Coded Elsewhere:

Agenesis of lacrimal ducts (LA14.11)

Congenital dacryocele (LA14.12)

Congenital agenesis of lacrimal punctum (LA14.13)

Congenital stenosis or stricture of lacrimal duct (LA14.14)

9A11.0

Eversion of lacrimal punctum

Inclusions:

Punctal ectropion

9A11.1

Canaliculitis

9A11.2

Dacryocystitis

9A11.3

Conjunctivochalasis

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9A11.4

Punctal stenosis

9A11.5

Nasolacrimal canalicular stenosis

9A11.6

Dacryolith

9A11.7

Nasolacrimal sac stenosis

9A11.8

Nasolacrimal duct obstruction

Coded Elsewhere:

Congenital stenosis or stricture of lacrimal duct (LA14.14)

9A11.Y

Other specified disorders of lacrimal drainage system

9A11.Z

Disorders of lacrimal drainage system, unspecified

9A1Y

Other specified disorders of lacrimal apparatus

9A1Z

Disorders of lacrimal apparatus, unspecified

Disorders of orbit (BlockL2‑9A2)

This refers to disorders of the cavity or socket of the skull in which the eye and its appendages are
situated. "Orbit" can refer to the bony socket, or it can also be used to imply the contents.

Coded Elsewhere:

Neoplasms of orbit

Orbital trauma

Structural developmental anomalies of orbit (LA14.2)

9A20

Displacement of eyeball

9A20.0

Axial displacement of eyeball

9A20.00

Outward displacement of eyeball

Inclusions:

Proptosis

Exophthalmos

9A20.01

Inward displacement of eyeball

Inclusions:

Enophthalmos

9A20.0Y

Other specified axial displacement of eyeball

9A20.0Z

Axial displacement of eyeball, unspecified

9A20.1

Non-Axial displacement of eyeball

9A20.Y

Other specified displacement of eyeball

9A20.Z

Displacement of eyeball, unspecified

9A21

Orbital infection
Coded Elsewhere:

Osteomyelitis of orbit (FB84.Y)

Hydatic cyst (9A23.1)

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Echinococcus infection of orbit (1F73.Y)

Myiasis of orbit (1G01.0)

9A21.0

Orbital cellulitis

Exclusions:

Streptococcal cellulitis of skin (1B70.1)

Staphylococcal cellulitis of skin (1B70.2)

9A21.1

Orbital subperiosteal abscess
A condition of the eye and adnexa, caused by an infection with a bacterial source.
This condition is characterised by a focal accumulation of purulent material in the
bones that support the globe, fever, crusting of the eye, swelling of the eye, or
proptosis. Confirmation is by identification of the bacterial agent.

9A21.2

Orbital abscess

9A21.3

Periostitis of orbit

9A21.Y

Other specified orbital infection

9A21.Z

Orbital infection, unspecified

9A22

Orbital inflammation

9A22.0

Dysthyroid orbitopathy

9A22.1

Diffuse orbital inflammation

9A22.2

Granulomatous orbital inflammation

9A22.Y

Other specified orbital inflammation

9A22.Z

Orbital inflammation, unspecified

9A23

Orbital cyst

9A23.0

Congenital orbital cyst

Coded Elsewhere:

Teratoma of orbit (2F36.3)

Dermoid cyst of eyelid (2F36.4)

9A23.1

Acquired orbital cyst

Coded Elsewhere:

Epidermoid cyst (EK70.0)

9A23.Z

Orbital cyst, unspecified

9A24

Bony deformity of orbit

9A24.0

Contraction of orbit

9A24.1

Expansion of orbit

9A24.2

Distortion of orbit

9A24.3

Enlargement of bony orbit

9A24.4

Exostosis of orbit

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9A24.Y

Other specified bony deformity of orbit

9A24.Z

Bony deformity of orbit, unspecified

9A25

Soft tissue deformity of orbit

9A25.0

Anophthalmic socket

9A25.1

Microphthalmic socket

9A25.2

Contracted socket

9A25.3

Oedema of orbit

9A25.4

Haemorrhage of orbit
This is the loss of blood or blood escaping from the circulatory system. This
diagnosis is of the cavity or socket of the skull in which the eye and its appendages
are situated. "Orbit" can refer to the bony socket, or it can also be used to imply the
contents.

9A25.5

Atrophy of soft tissue of orbit

9A25.Y

Other specified soft tissue deformity of orbit

9A25.Z

Soft tissue deformity of orbit, unspecified

9A26

Combined bony and soft tissue deformity of orbit
Coded Elsewhere:

Hypertelorism (LB71.1)

9A2Y

Other specified disorders of orbit

9A2Z

Disorders of orbit, unspecified

9A4Y

Other specified disorders of the ocular adnexa or orbit

9A4Z

Disorders of the ocular adnexa or orbit, unspecified

Disorders of the eyeball - anterior segment (BlockL1‑9A6)

This refers to any disorders of the front third of the eye that includes the structures in front of the
vitreous humour: the cornea, iris, ciliary body, and lens.

Coded Elsewhere:

Structural disorders of the pupil (LA11.6)

Developmental anomalies of anterior segment (LA11.Y)

Disorders of conjunctiva (BlockL2‑9A6)

Coded Elsewhere:

Neoplasms of conjunctiva

9A60

Conjunctivitis
Exclusions:

keratoconjunctivitis (BlockL2‑9A7)

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Coded Elsewhere:

Trachoma (1C23)

Viral conjunctivitis (1D84)

Neonatal conjunctivitis or dacryocystitis (KA65.0)

9A60.0

Papillary conjunctivitis

9A60.00

Giant papillary conjunctivitis
Giant papillary conjunctivitis is a nonallergic hypersensitivity inflammation of the
ocular surface, most frequently to contact lenses, ocular prostheses, postoperative
sutures, and scleral buckles.

9A60.01

Acute atopic conjunctivitis
This is the allergic inflammation of the conjunctiva (mucous membrane that covers
the posterior surface of the eyelids and the anterior pericorneal surface of the
eyeball) of the immediate type, due to airborne allergens such as pollens, dusts,
spores, and animal hair.

9A60.02

Allergic conjunctivitis
Allergic conjunctivitis is an IgE-mediated response due to the exposure of seasonal
or perennial allergens in sensitized patients. The allergen-induced inflammatory
response of the conjunctiva results in the release of histamine and other mediators.
Symptoms consist of redness (mainly due to vasodilation of the peripheral small
blood vessels), oedema (swelling) of the conjunctiva, itching, and increased
lacrimation (production of tears).

9A60.0Y

Other specified papillary conjunctivitis

9A60.0Z

Papillary conjunctivitis, unspecified

9A60.1

Follicular conjunctivitis

Coded Elsewhere:

Chlamydial conjunctivitis (1C20)

Herpes simplex keratoconjunctivitis (1F00.1Y)

Zoster keratoconjunctivitis (1E91.1)

Keratoconjunctivitis due to Acanthamoeba (1F50)

Keratoconjunctivitis due to adenovirus (1D84.0)

9A60.2

Cicatrizing conjunctivitis

9A60.3

Mucopurulent conjunctivitis
These are infections of the conjunctiva, containing mucus and pus, by several
species such as Haemophilus, Streptococcus, Neisseria, and Chlamydia.

9A60.30

Ulceration of conjunctiva

9A60.31

Abscess of conjunctiva

9A60.32

Conjunctivitis due to Koch-Weeks bacillus

9A60.33

Acute epidemic conjunctivitis

9A60.3Y

Other specified mucopurulent conjunctivitis

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9A60.3Z

Mucopurulent conjunctivitis, unspecified

9A60.4

Blepharoconjunctivitis

9A60.5

Vernal keratoconjunctivitis
Vernal keratoconjunctivitis is a persistent and severe form of ocular allergy that
affects children and young adults, usually in warm climates. Vernal
keratoconjunctivitis typically appears in boys between the ages of 4–12 years. The
typical symptoms are intense itching, tearing, and photophobia. Disease
exacerbation can be triggered either by allergen re-exposure or by nonspecific
stimuli such as sunlight, wind, and dust. The tarsal form is characterised by
irregularly sized hypertrophic papillae, leading to a cobblestone appearance of the
upper tarsal plate. The limbal form is characterised by transient, multiple limbal, or
conjunctival gelatinous yellow-grey infiltrates superposed with white points or
deposits, known as Horner–Trantas dots and papillae at the limbus.

9A60.6

Serous conjunctivitis, except viral

9A60.Y

Other specified conjunctivitis

9A60.Z

Conjunctivitis, unspecified

9A61

Certain specified disorders of conjunctiva
Exclusions:

keratoconjunctivitis (BlockL2‑9A7)

Coded Elsewhere:

Conjunctival blebitis after glaucoma surgery (9D23)

Complications with glaucoma drainage devices (9D24)

Injury of conjunctiva or corneal abrasion without mention of

foreign body (NA06.4)

Foreign body in conjunctival sac (ND70.1)

9A61.0

Pingueculae

9A61.1

Pterygium

Exclusions:

Pseudopterygium of conjunctiva (9A61.2)

9A61.2

Pseudopterygium of conjunctiva

9A61.3

Conjunctival scars
These are cicatrices of the mucous membrane that lines the inner surface of the
eyelid and the exposed surface of the eyeball that occur due to various reasons
such as trauma, infection or allergy.

9A61.4

Conjunctival vascular disorders
Benign cysts which often appear as small, clear, fluid-filled inclusions of
conjunctival epithelium whose goblet cells secrete into the cyst and not onto the
surface.

9A61.40

Vascular abnormalities of conjunctiva

Coded Elsewhere:

Conjunctival haemangioma or haemolymphangioma (2E81.01)

9A61.4Y

Other specified conjunctival vascular disorders

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9A61.4Z

Conjunctival vascular disorders, unspecified

9A61.5

Conjunctival or subconjunctival haemorrhage
A conjunctival haemorrhage is a small haematoma clearly delimited on the
conjunctiva itself resulting from a direct blow on the eye. Subconjunctival
haemorrhage extends from the orbit, forward and deep to the conjunctiva with no
posterior limit.

9A61.6

Conjunctival or subconjunctival degenerations or deposits
These are the conjunctival/subconjunctival accumulation of some materials and
gradual deterioration with impairment or loss of function, caused by injury, disease,
or aging.

Coded Elsewhere:

Vitamin A deficiency with conjunctival xerosis (5B55.1)

Vitamin A deficiency with conjunctival xerosis or Bitot's spots

(5B55.2)

9A61.Z

Certain specified disorders of conjunctiva, unspecified

9A62

Mucous membrane pemphigoid with ocular involvement

Mucous membrane pemphigoid (MMP) involving the conjunctivae is also known as
ocular pemphigoid. This may be confined to the conjunctivae or may be associated
with involvement of other sites as well. Its importance lies in its potential to cause
loss of vision and it may thus warrant more aggressive therapy than would be
considered for MMP of other sites.

Coded Elsewhere:

Chronic cicatrizing conjunctivitis, ocular cicatricial pemphigoid

(9A60.2)

9A6Y

Other specified disorders of conjunctiva

9A6Z

Disorders of conjunctiva, unspecified

Disorders of the cornea (BlockL2‑9A7)

This refers to disorders of the transparent front part of the eye that covers the iris, pupil, and anterior
chamber. The cornea, with the anterior chamber and lens, refracts light, with the cornea accounting
for approximately two-thirds of the eye's total optical power.

Coded Elsewhere:

Neoplasms of the cornea

9A70

Hereditary corneal dystrophies

The term corneal dystrophy embraces a heterogeneous group of bilateral
genetically determined non-inflammatory corneal diseases that are usually
restricted to the cornea. The designation is imprecise but remains in vogue because
of its clinical value.

Coded Elsewhere:

X-linked ichthyosis (EC20.01)

Cornea plana (LA11.1)

Megalocornea (LA11.1)

Microcornea (LA11.1)

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9A70.0

Endothelial corneal dystrophy

9A70.Y

Other specified hereditary corneal dystrophies

9A70.Z

Hereditary corneal dystrophies, unspecified

9A71

Infectious keratitis
Coded Elsewhere:

Herpes simplex keratitis (1F00.10)

9A72

Traumatic keratitis
Exclusions:

Foreign body in cornea (ND70.0)

9A73

Exposure keratitis

This is an exposure condition in which the eye's cornea, the front part of the eye,
becomes inflamed. The condition is often marked by moderate to intense pain and
usually involves impaired eyesight. May cause feelings of scratching each time
individual blinks eye.

9A74

Neurotrophic keratitis

Coding Note:

Code aslo the casusing condition

9A75

Autoimmune keratitis

9A76

Corneal ulcer

Loss of epithelial tissue from the surface of the cornea due to progressive erosion
and necrosis of the tissue. It is often caused by bacterial, fungal, or viral infection.

Coding Note:

Code aslo the casusing condition

9A77

Corneal scars or opacities

Corneal opacity occurs when the cornea is scarred by a variety of infectious and
inflammatory eye diseases. These scars stop light from passing through the cornea
to the retina and may cause the cornea which is normally transparent to appear
white or clouded over.

Coded Elsewhere:

Anterior corneal pigmentations (9A78.1)

Posterior corneal pigmentations (9A78.1)

Stromal corneal pigmentations (9A78.1)

9A77.0

Contact lens-associated corneal infiltrates

9A77.1

Adherent leukoma
This is a white tumour of the cornea enclosing a prolapsed adherent iris.

9A77.Y

Other specified corneal scars or opacities

9A77.Z

Corneal scars or opacities, unspecified

9A78

Certain specified disorders of cornea
Coded Elsewhere:

Injury of conjunctiva or corneal abrasion without mention of

foreign body (NA06.4)

Ocular laceration or rupture with prolapse or loss of intraocular

16

ICD-11 MMS – 09/2020

tissue, unilateral (NA06.87)

Ocular laceration without prolapse or loss of intraocular tissue,

unilateral (NA06.8D)

Ocular laceration or rupture with prolapse or loss of intraocular

tissue, bilateral (NA06.88)

Ocular laceration without prolapse or loss of intraocular tissue,

bilateral (NA06.8E)

Foreign body in cornea (ND70.0)

Chemical burn of cornea or conjunctival sac (NE00)

9A78.0

Corneal neovascularization

9A78.1

Corneal pigmentations or deposits

9A78.2

Corneal oedema

9A78.20

Bullous keratopathy
This is the maximum stage of corneal oedema.

It is a pathological condition in which small vesicles, or bullae, are formed in the
cornea due to endothelial dysfunction. In a healthy cornea, endothelial cells keeps
the tissue from excess fluid absorption, pumping it back into the aqueous humour.
When affected by some reason, such as Fuchs' dystrophy or a trauma during
cataract removal, endothelial cells suffer mortality or damage. The corneal
endothelial cells normally do not undergo mitotic cell division, and cell loss results
in permanent loss of function.

9A78.21

Secondary corneal oedema

9A78.2Y

Other specified corneal oedema

9A78.2Z

Corneal oedema, unspecified

9A78.3

Changes in corneal membranes

9A78.4

Corneal degeneration

Exclusions:

Mooren ulcer (9A76)

Coded Elsewhere:

Vitamin A deficiency with corneal xerosis (5B55.3)

Vitamin A deficiency with corneal ulceration or keratomalacia

(5B55.4)

Vitamin A deficiency with xerophthalmic scars of cornea or

blindness (5B55.5)

9A78.5

Corneal deformities

Coded Elsewhere:

Structural developmental anomalies of cornea (LA11.1)

9A78.50

Keratoconus
Keratoconus is a noninflammatory, often bilateral, corneal dystrophy characterised
by progressive cone-shaped bulging and thinning of the cornea.

Coding Note:

Code aslo the casusing condition

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9A78.51

Corneal staphyloma

9A78.5Y

Other specified corneal deformities

9A78.5Z

Corneal deformities, unspecified

9A78.6

Anaesthesia of cornea
This is the condition of having sensation (including the feeling of pain) blocked or
temporarily taken away, of the transparent front part of the eye that covers the iris,
pupil, and anterior chamber.

9A78.7

Hypoesthesia of cornea
This refers to a reduced sense of touch or sensation, or a partial loss of sensitivity
to sensory stimuli, of the transparent front part of the eye that covers the iris, pupil,
and anterior chamber.

9A78.8

Recurrent erosion of cornea

9A78.9

Corneal abscess

9A78.A

Sclerosing keratitis

9A78.Z

Certain specified disorders of cornea, unspecified

9A79

Keratoconjunctivitis sicca

9A7Y

Other specified disorders of the cornea

9A7Z

Disorders of the cornea, unspecified

Disorders of the anterior chamber (BlockL2‑9A8)

Coded Elsewhere:

Hypopyon (9A96.4)

Retained foreign body in anterior chamber of eye (NA06.2)

9A80

Hyphaema
Exclusions:

traumatic hyphaema (NA06.9)

9A81

Parasites in the anterior chamber of the eye

Coding Note:

Code aslo the casusing condition

9A82

Cyst in the anterior chamber of the eye

9A83

Flat anterior chamber hypotony of eye

9A8Y

Other specified disorders of the anterior chamber

9A8Z

Disorders of the anterior chamber, unspecified

Disorders of the anterior uvea (BlockL2‑9A9)

Coded Elsewhere:

Congenital malformations of the uvea

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ICD-11 MMS – 09/2020

Neoplasms of the iris

Neoplasms of the ciliary body

Corectopia (LA11.Y)

Polycoria (LA11.Y)

9A90

Degeneration of iris or ciliary body

9A90.0

Disorders of chamber angle
This refers to the change of tissue to a lower or less functionally active form, of the
fluid-filled space inside the eye between the iris and the cornea's innermost surface,
the endothelium.

9A90.1

Degeneration of iris
This refers to the change of tissue to a lower or less functionally active form, of the
thin, circular structure in the eye, responsible for controlling the diameter and size of
the pupil and thus the amount of light reaching the retina. The color of the iris is
often referred to as "eye color."

9A90.2

Iris atrophy

9A90.Y

Other specified degeneration of iris or ciliary body

9A90.Z

Degeneration of iris or ciliary body, unspecified

9A91

Cyst of iris, ciliary body or anterior chamber
Coded Elsewhere:

Cyst in the anterior chamber of the eye (9A82)

9A92

Persistent pupillary membranes

9A93

Adhesions or disruptions of iris or ciliary body

This refers to adhesions and disruptions of the thin, circular structure in the eye,
responsible for controlling the diameter and size of the pupil and thus the amount
of light reaching the retina. The colour of the iris is often referred to as "eye colour."
It is also of the circumferential tissue inside the eye composed of the ciliary muscle
and ciliary processes. It is triangular in horizontal section and is coated by a double
layer, the ciliary epithelium.

Exclusions:

Corectopia (LA11)

9A94

Certain specified disorders of iris or ciliary body

9A94.0

Rubeosis of iris

9A94.1

Floppy iris syndrome
This is a complication that may occur during cataract extraction in certain patients.
This syndrome is characterised by a flaccid iris which billows in response to
ordinary intraocular fluid currents, a propensity for this floppy iris to prolapse
towards the area of cataract extraction during surgery, and progressive
intraoperative pupil constriction despite standard procedures to prevent this.

9A94.2

Plateau iris syndrome

9A94.Y

Other disorders of iris and ciliary body

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9A96

Anterior uveitis

Coding Note:

Code aslo the casusing condition

9A96.0

Anterior uveitis not associated with systemic conditions

9A96.1

Anterior uveitis associated with systemic conditions

Coding Note:

Code aslo the casusing condition

Coded Elsewhere:

Sarcoid associated anterior uveitis (4B20.4)

9A96.2

Infection-associated anterior uveitis

Coding Note:

Code aslo the casusing condition

Coded Elsewhere:

Gonococcal anterior uveitis (1A72.4)

Zoster anterior uveitis (1E91.1)

Secondary syphilitic anterior uveitis (1A61.4)

Tuberculous anterior uveitis (1B12.1)

Chronic tuberculous iridocyclitis (1B12.1)

Syphilitic uveitis (1A62.20)

9A96.3

Primary anterior uveitis
This refers to primary inflammation of the uvea. The uvea consists of the middle,
pigmented, vascular structures of the eye and includes the iris, ciliary body, and
choroid.

9A96.4

Hypopyon
Hypopyon is inflammatory cells in the anterior chamber of eye. It is a leukocytic
exudate, seen in the anterior chamber, usually accompanied by redness of the
conjunctiva and the underlying episclera. It is a sign of inflammation of the anterior
uvea and iris, i.e. iritis, which is a form of anterior uveitis. The exudate settles at the
bottom due to gravity.

9A96.Y

Other specified anterior uveitis

Coding Note:

Code aslo the casusing condition

9A96.Z

Anterior uveitis, unspecified

Coding Note:

Code aslo the casusing condition

9A9Y

Other specified disorders of the anterior uvea

9A9Z

Disorders of the anterior uvea, unspecified

Functional disorders of the pupil (BlockL2‑9B0)

9B00

Disorders of the afferent pupillary system

9B00.0

Relative afferent pupillary defects

9B00.1

Amaurotic pupillary reaction

20

ICD-11 MMS – 09/2020

9B00.2

Paradoxical pupillary reaction to light or darkness

9B00.3

Wernicke pupils

9B00.Y

Other specified disorders of the afferent pupillary system

9B00.Z

Disorders of the afferent pupillary system, unspecified

9B01

Disorders of the efferent pupillary system
Coded Elsewhere:

Horner syndrome (8D8A.1)

Horner syndrome, acquired (8D8A.1)

Horner syndrome, congenital (8D8A.1)

9B01.0

Physiologic anisocoria

9B01.1

Parasympathoparetic pupils
Damage to the parasympathetic outflow to the iris sphincter muscle

Coded Elsewhere:

Third nerve palsy (9C81.0)

9B01.2

Pharmacologic inhibition of the parasympathetic pathway

9B01.3

Iris sphincter disorders
This refers to disorders of the muscle in the part of the eye called the iris. It
encircles the pupil of the iris, appropriate to its function as a constrictor of the pupil.

9B01.4

Pharmacologic parasympathicotonic pupils
Pharmacologic stimulation of the parasympathetic pathway

9B01.5

Pharmacologic sympathoparetic pupils

9B01.6

Sympathotonic pupils

9B01.7

Episodic unilateral mydriasis

9B01.Y

Other specified disorders of the efferent pupillary system

9B01.Z

Disorders of the efferent pupillary system, unspecified

9B02

Light-near dissociations

9B02.0

Argyll Robertson pupil
These are bilateral small pupils that constrict when the patient focuses on a near
object but do not constrict when exposed to bright light (they do not “react” to light).

Coding Note:

Code aslo the casusing condition

Coded Elsewhere:

Syphilitic Argyll Robertson pupil (1A62.01)

9B02.1

Pregeniculate light-near dissociations

9B02.2

Mesencephalic light-near dissociations

9B02.Y

Other specified light-near dissociations

9B02.Z

Light-near dissociations, unspecified

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9B0Y

Other specified functional disorders of the pupil

9B0Z

Functional disorders of the pupil, unspecified

Disorders of lens (BlockL2‑9B1)

Coded Elsewhere:

Structural developmental anomalies of lens or zonula (LA12)

Presence of intraocular lens (QB51.2)

9B10

Cataract

9B10.0

Age-related cataract
A senile cataract is a clouding of the lens of the eye, which impedes the passage of
light, related to ageing, and that occurs usually starting from the age of 40.

Exclusions:

capsular glaucoma with pseudoexfoliation of lens (9C61.0)

9B10.00

Coronary age-related cataract

9B10.01

Punctate age-related cataract

9B10.02

Mature age-related cataract
This is a mature age-related clouding of the lens inside the eye which leads to a
decrease in vision. It is the most common cause of blindness and is conventionally
treated with surgery. Visual loss occurs because opacification of the lens obstructs
light from passing and being focused on to the retina at the back of the eye.

9B10.0Y

Other specified age-related cataract

9B10.0Z

Age-related cataract, unspecified

9B10.1

Infantile or juvenile cataract
A cataract is clouding of the lens of the eye, which impedes the passage of light.

Exclusions:

Congenital cataract (LA12.1)

9B10.10

Combined forms of infantile and juvenile cataract

9B10.1Y

Other specified infantile or juvenile cataract

9B10.1Z

Infantile or juvenile cataract, unspecified

9B10.2

Certain specified cataracts
A cataract is clouding of the lens of the eye, which impedes the passage of light.

Exclusions:

Congenital cataract (LA12.1)

9B10.20

Traumatic cataract
Partial or complete opacity on or in the lens or capsule of one or both eyes,
impairing vision or causing blindness. The many kinds of cataract are classified by
their morphology (size, shape, location) or etiology (cause and time of occurrence)
resulting from or following injury.

9B10.21

Diabetic cataract

22

ICD-11 MMS – 09/2020

This refers to an unspecified group of metabolic diseases in which a person has
high blood sugar, either because the pancreas does not produce enough insulin, or
because cells do not respond to the insulin that is produced. This diagnosis is with
diabetic cataract.

Coding Note:

Always assign an additional code for diabetes mellitus.

9B10.22

After-cataract
This is a clouding of the lens of the eye, which impedes the passage of light
resulting from disease, degeneration, or from surgery.

Inclusions:

Secondary cataract

Soemmerring ring

9B10.23

Subcapsular glaucomatous flecks

Coding Note:

Code aslo the casusing condition

9B10.2Y

Other specified cataracts

9B10.Z

Cataract, unspecified

9B11

Certain specified disorders of lens
Exclusions:

congenital lens malformations (LA12)

Cataract lens fragments in eye following cataract surgery

(9D21)

Coded Elsewhere:

Presence of intraocular lens (QB51.2)

9B11.0

Aphakia

9B11.1

Dislocation of lens

9B11.Y

Other disorders of lens

9B1Z

Disorders of lens, unspecified

9B3Y

Other specified disorders of the eyeball - anterior segment

9B3Z

Disorders of the eyeball - anterior segment, unspecified

Disorders of the eyeball - posterior segment (BlockL1‑9B5)

This refers to disorders of the back two-thirds of the eye that includes the anterior hyaloid membrane
and all of the optical structures behind it: the vitreous humour, retina, choroid, and optic nerve.

Disorders of sclera (BlockL2‑9B5)

Coded Elsewhere:

Blue sclera (LA11.0)

9B50

Episcleritis

Episcleritis is a benign, self-limiting inflammatory disease affecting part of the eye
called the episclera. The episclera is a thin layer of tissue that lies between the

INTERNATIONAL CLASSIFICATION OF DISEASES -

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ICD-11 MMS – 09/2020

23

conjunctiva and the connective tissue layer that forms the white of the eye (sclera).
Episcleritis is a common condition, and is characterised by the abrupt onset of mild
eye pain and redness.

Coded Elsewhere:

Tuberculous episcleritis (1B12.1)

Late syphilitic episcleritis (1A62.20)

9B51

Scleritis

Inflammation of the white, opaque, fibrous, outer tunic of the eyeball. Can be
associated with uveitis.

Coded Elsewhere:

Zoster scleritis (1E91.1)

9B52

Scleral staphyloma
Exclusions:

degenerative myopia (9B76)

9B5Y

Other specified disorders of sclera

9B5Z

Disorders of sclera, unspecified

Disorders of the choroid (BlockL2‑9B6)

Inclusions:

Disorders of posterior uvea

Coded Elsewhere:

Neoplasms of choroid

Congenital malformations of choroid (LA13.6)

9B60

Choroidal degeneration
Exclusions:

angioid streaks (9B78.3)

9B61

Choroidal dystrophy
Exclusions:

ornithinaemia (5C50.9)

9B62

Chorioretinal scars

9B63

Choroidal haemorrhage or rupture
Coded Elsewhere:

Choroidal rupture (NA06.61)

9B64

Choroidal detachment

9B65

Choroiditis

Coding Note:

Code aslo the casusing condition

Inclusions:

Posterior uveitis

9B65.0

Noninfectious posterior choroiditis

Coded Elsewhere:

Ocular Behçet disease (4A62)

9B65.1

Infectious posterior choroiditis

Coded Elsewhere:

Late syphilitic posterior uveitis (1A62.20)

Toxoplasma posterior uveitis (1F57.3)

24

ICD-11 MMS – 09/2020

Tuberculous posterior uveitis (1B12.1)

9B65.2

Chorioretinal inflammation

Coded Elsewhere:

Toxoplasma chorioretinitis (1F57.3)

Tuberculous chorioretinitis (1B12.1)

Late congenital syphilitic chorioretinitis (1A60.2)

9B65.Z

Choroiditis, unspecified

Coding Note:

Code aslo the casusing condition

9B66

Intermediate choroiditis

This is a form of uveitis localised to the vitreous and peripheral retina. Primary sites
of inflammation include the vitreous of which other such entities as pars planitis,
posterior cyclitis, and hyalitis are encompassed. Intermediate uveitis may either be
an isolated eye disease or associated with the development of a systemic disease
such as multiple sclerosis or sarcoidosis.

9B66.0

Noninfectious intermediate choroiditis
This is a non-infectious form of uveitis localised to the vitreous and peripheral retina.
Primary sites of inflammation include the vitreous of which other such entities as
pars planitis, posterior cyclitis, and hyalitis are encompassed. Intermediate uveitis
may either be an isolated eye disease or associated with the development of a
systemic disease such as multiple sclerosis or sarcoidosis.

9B66.1

Infectious intermediate choroiditis
This is a infectious form of uveitis localised to the vitreous and peripheral retina.
Primary sites of inflammation include the vitreous of which other such entities as
pars planitis, posterior cyclitis, and hyalitis are encompassed. Intermediate uveitis
may either be an isolated eye disease or associated with the development of a
systemic disease such as multiple sclerosis or sarcoidosis.

9B66.Z

Intermediate choroiditis, unspecified

9B6Y

Other specified disorders of the choroid

9B6Z

Disorders of the choroid, unspecified

Disorders of the retina (BlockL2‑9B7)

Coded Elsewhere:

Certain congenital malformations of posterior segment of eye (LA13.8)

Neoplasms of retina

Traumatic injuries of the retina (NA06.6)

Renal retinitis in chronic kidney disease, stage 5 (GB61.5)

Presence of retina Implant (QB51.Y)

Coats disease (LD21.Y)

9B70

Inherited retinal dystrophies
Coded Elsewhere:

Sjögren-Larsson syndrome (5C52.03)

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Usher syndrome (LD2H.4)

Asphyxiating thoracic dystrophy (LD24.B1)

9B71

Retinopathy

Coding Note:

Code aslo the casusing condition

9B71.0

Diabetic retinopathy
A condition characterised as a disease of the retina (retinopathy) involving damage
to the small blood vessels in the retina which is due to chronically high blood
glucose levels in people with diabetes.

Coding Note:

Always assign an additional code for diabetes mellitus.

9B71.00

Nonproliferative diabetic retinopathy

Coding Note:

Code aslo the casusing condition

9B71.01

Proliferative diabetic retinopathy
This is proliferative retinopathy (damage to the retina) caused by complications of
diabetes, which can eventually lead to blindness. It is an ocular manifestation of
diabetes, a systemic disease, which affects up to 80 percent of all patients who
have had diabetes for 10 years or more.

Always assign an additional code for the type of diabetes mellitus.

Coding Note:

Code aslo the casusing condition

9B71.02

Diabetic macular oedema

Coding Note:

Code aslo the casusing condition

9B71.0Z

Diabetic retinopathy, unspecified

Coding Note:

Always assign an additional code for diabetes mellitus.

9B71.1

Hypertensive retinopathy

Coding Note:

Code aslo the casusing condition

9B71.2

Radiation retinopathy
Radiation retinopathy is damage to retina due to exposure to ionizing radiation.
Radiation retinopathy has a delayed onset, typically after months or years of
radiation, and is slowly progressive. In general, radiation retinopathy is seen around
18 months after treatment with external-beam radiation and with brachytherapy.

9B71.3

Retinopathy of prematurity
Retinopathy of prematurity is a vasoproliferative disorder that affects extremely
premature infants potentially leading to severe visual impairment or blindness.
Exposure of newborn premature infants to hyperoxia down regulates retinal
vascular endothelial growth factor, Blood vessels constrict and can become
obliterated, resulting in delays of normal retinal vascular development. Low birth
weight, young gestational age, and severity of illness (e.g. respiratory distress
syndrome, bronchopulmonary dysplasia, sepsis) are associated factors. It primarily
occurs in extremely low birth weight infants because of the cessation of normal
retinal vascular maturation.

26

ICD-11 MMS – 09/2020

Coding Note:

Code aslo the casusing condition

Inclusions:

Retrolental fibroplasia

9B71.4

Paraneoplastic retinopathy
Paraneoplastic retinopathies results from a targeted attack on the retina due to a
tumour immune response initiated by onco-neural antigens derived from systemic
cancer. Patients usually present after cancer diagnosis with progressive visual
dimming and photopsias but dysfunction of rods (impaired dark adaption and
peripheral vision loss) and cones (decreased visual acuity, colour dysfunction,
photosensitivity and glare) may also occur. Symptoms are often worse than clinical
signs. Other causes of retinopathy should be excluded. Multiple anti-retinal
autoantibodies (e.g. anti-recoverin antibodies) are described although their
significance is uncertain. Two major subsets are recognised: cancer-associated-
retinopathy (most commonly small-cell-lung-cancer) and melanoma-associated-
retinopathy.

Associated neural autoantibodies include:

CRMP5 (anti-CV2) (collapsin response mediator protein 5 - anti CV2); anti-recoverin
autoantibodies; and alpha-enolase autoantibodies.

Coding Note:

Code aslo the casusing condition

9B71.40

Melanoma associated retinopathy

9B71.4Y

Other specified paraneoplastic retinopathy

Coding Note:

Code aslo the casusing condition

9B71.4Z

Paraneoplastic retinopathy, unspecified

Coding Note:

Code aslo the casusing condition

9B71.5

Autoimmune retinopathy
Autoimmune retinopathies are immune-mediated inflammatory disorders of the
retina that differ from paraneoplastic retinopathies in the lack of association with
cancer. Patients present with progressive visual loss and dysfunction of rods
(impaired dark adaption and peripheral vision problems) and cones (visual acuity,
colour dysfunction, photosensitivity and glare) may occur. The symptoms are often
worse than the clinical signs on fundoscopy. Multiple anti-retinal autoantibodies
(e.g. anti-recoverin antibodies) are described although their significance is uncertain.
Autoimmune retinopathy is a diagnosis of exclusion and other causes of
retinopathy need to be ruled out, while the potential role of immunotherapy remains
uncertain.

Associated neural autoantibodies include:

anti-recoverin autoantibodies; alpha-enolase autoantibodies; anti-transducin
autoantibodies;

Coding Note:

Code aslo the casusing condition

9B71.Y

Other specified retinopathy

Coding Note:

Code aslo the casusing condition

9B71.Z

Retinopathy, unspecified

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Coding Note:

Code aslo the casusing condition

9B72

Inflammatory diseases of the retina

This refers to inflammatory diseases of light-sensitive layer of tissue, lining the
inner surface of the eye. The optics of the eye create an image of the visual world
on the retina, which serves much the same function as the film in a camera.

Coded Elsewhere:

Retinal vasculitis (9B78.12)

9B72.0

Viral retinitis

9B72.00

Cytomegaloviral retinitis
This is an inflammation of the eye's retina that can lead to blindness. This is a DNA
virus in the family Herpesviridae known for producing large cells with nuclear and
cytoplasmic inclusions. Such inclusions are called an "owl's eye" effect.

9B72.01

HIV retinitis

9B72.0Y

Other specified viral retinitis

9B72.0Z

Viral retinitis, unspecified

9B72.Y

Other specified inflammatory diseases of the retina

9B72.Z

Inflammatory diseases of the retina, unspecified

9B73

Retinal detachments or breaks

Retinal breaks are full thickness openings in the neurosensory retina that can be in
the form of a hole, a tear or a retinal dialysis. Retinal detachment is a condition in
which the retina peels away from its underlying layer of support tissue.

Exclusions:

detachment of retinal pigment epithelium (9B78.6)

9B73.0

Retinal detachment with retinal break

Inclusions:

Rhegmatogenous retinal detachment

9B73.1

Retinoschisis

9B73.10

Adult retinoschisis

9B73.11

Juvenile retinoschisis
X-linked retinoschisis is a genetic ocular disease that is characterised by reduced
visual acuity in males due to juvenile macular degeneration.

9B73.1Y

Other specified retinoschisis

9B73.1Z

Retinoschisis, unspecified

9B73.2

Retinal cysts
Retinoschisis is an eye disease characterised by the abnormal splitting of the
retina's neurosensory layers. A retinal cyst is a closed sac, having a distinct
membrane and division compared to the nearby tissue in retina that can either be
congenital or acquired.

Exclusions:

congenital retinoschisis (LA13.3)

28

ICD-11 MMS – 09/2020

Microcystoid degeneration of retina (9B78.4)

9B73.3

Serous retinal detachment
This occurs due to inflammation, injury or vascular abnormalities that results in fluid
accumulating underneath the retina without the presence of a hole, tear, or break.

Exclusions:

Central serous chorioretinopathy (9B75.2)

9B73.4

Retinal breaks without detachment

Exclusions:

Chorioretinal scars after surgery for detachment (9D22)

peripheral retinal degeneration without break (9B78.4)

9B73.Y

Other specified retinal detachments or breaks

9B73.Z

Retinal detachments or breaks, unspecified

9B74

Retinal vascular occlusions

These are obstruction or closure of retinal vascular structures.

Exclusions:

amaurosis fugax (9D51)

9B74.0

Retinal artery occlusions

9B74.1

Retinal venous occlusions

9B74.2

Combined arterial and vein occlusion

9B74.Y

Other specified retinal vascular occlusions

9B74.Z

Retinal vascular occlusions, unspecified

9B75

Macular disorders

9B75.0

Age related macular degeneration
Age-related macular degeneration (ARMD) is defined as an ocular disease leading
to loss of central vision in the elderly, and characterised by primary and secondary
damage of macular retinal pigment epithelial (RPE) cells, resulting in formation of
drusen (deposits lying beneath the RPE), choroidal neovascularization (CNV), and
atrophy of photoreceptors and choriocapillaris layer of the choroidea.

Coded Elsewhere:

Small drusen of the macula (MC20.1)

9B75.00

Early age related macular degeneration
consists of a combination of multiple small drusen, few intermediate drusen (63 to
124 microns in diameter), or RPE abnormalities.

9B75.01

Intermediate age related macular degeneration
consists of extensive intermediate drusen, at least one large druse (>=125 microns
in diameter), or geographic atrophy not involving the centre of the fovea

9B75.02

Advanced age related macular degeneration

9B75.0Y

Other specified age related macular degeneration

9B75.0Z

Age related macular degeneration, unspecified

INTERNATIONAL CLASSIFICATION OF DISEASES -

Mortality and Morbidity Statistics

ICD-11 MMS – 09/2020

29

9B75.1

Non-traumatic macular hole

9B75.2

Central serous chorioretinopathy
This is an eye disease which causes visual impairment, often temporary, usually in
one eye. When the disorder is active it is characterised by leakage of fluid under the
retina that has a propensity to accumulate under the central macula.

9B75.3

Macular telangiectasia

9B75.Y

Other specified macular disorders

9B75.Z

Macular disorders, unspecified

9B76

Degenerative high myopia

9B77

Eales disease

Eales disease is a retinal vasculopathy that presents as an inflammatory stage with
retinal periphlebitis affecting especially peripheral retina, then an ischemic stage
with sclerosis of retinal veins, and finally a proliferative stage characterised by
neovascularization, haemorrhage and retinal detachment.

9B78

Certain specified retinal disorders
Coded Elsewhere:

Double heterozygous sickling disorders with retinopathy

(3A51.3)

Retinal dystrophy in GM2 gangliosidosis (5C56.00)

9B78.0

Retinal vasculopathy and cerebral leukodystrophy
Retinal vasculopathy and cerebral leukodystrophy is an inherited group of small
vessel diseases comprised of cerebroretinal vasculopathy, hereditary vascular
retinopathy and hereditary endotheliopathy with retinopathy, nephropathy and
stroke (HERNS); all exhibiting progressive visual impairment as well as variable
cerebral dysfunction.

Coded Elsewhere:

HERNS syndrome (LD2F.1Y)

9B78.1

Background retinopathy and retinal vascular changes
Background retinopathy is the earliest visible change to the retina in diabetes,
characterised by some retinal vascular changes such as the capillaries in the retina
become blocked, they may bulge slightly (microaneurysm) and may leak blood or
fluid.

9B78.10

Changes in retinal vascular appearance

9B78.11

Exudative retinopathy

9B78.12

Retinal vasculitis

9B78.13

Retinal telangiectasis

9B78.1Y

Other specified background retinopathy and retinal vascular changes

9B78.1Z

Background retinopathy and retinal vascular changes, unspecified

30

ICD-11 MMS – 09/2020

9B78.2

Other proliferative retinopathy

Exclusions:

proliferative vitreo-retinopathy with retinal detachment (9B73)

Proliferative diabetic retinopathy (9B71.01)

9B78.3

Degeneration of macula or posterior pole

9B78.30

Reticular pseudodrusen
Histologically located above the retinal pigment epithelium, this finding is often
associated with other retinal disease.

9B78.3Y

Other specified degeneration of macula or posterior pole

9B78.3Z

Degeneration of macula or posterior pole, unspecified

9B78.4

Peripheral retinal degeneration

Exclusions:

with retinal break (9B73.4)

9B78.5

Retinal haemorrhage

Exclusions:

Traumatic retinal haemorrhage (NA06.7)

9B78.6

Separation of retinal layers

Inclusions:

Detachment of retinal pigment epithelium

9B78.60

Serous detachment of retinal pigment epithelium
This refers to the serous detachment of the pigmented cell layer just outside the
neurosensory retina that nourishes retinal visual cells, and is firmly attached to the
underlying choroid and overlying retinal visual cells.

9B78.61

Haemorrhagic detachment of retinal pigment epithelium
This refers to the haemorrhagic detachment of the pigmented cell layer just outside
the neurosensory retina that nourishes retinal visual cells, and is firmly attached to
the underlying choroid and overlying retinal visual cells.

9B78.6Y

Other specified separation of retinal layers

9B78.6Z

Separation of retinal layers, unspecified

9B78.7

Retinal oedema

9B78.8

Retinal ischaemia

Coding Note:

Code aslo the casusing condition

9B78.9

Retinal atrophy
This is a group of genetic diseases and is characterised by the bilateral
degeneration of the retina, causing progressive vision loss culminating in blindness.

9B7Y

Other specified disorders of the retina

9B7Z

Disorders of the retina, unspecified

INTERNATIONAL CLASSIFICATION OF DISEASES -

Mortality and Morbidity Statistics

ICD-11 MMS – 09/2020

31

Disorders of the vitreous body (BlockL2‑9B8)

Any condition of the transparent, semigelatinous substance that fills the cavity behind the crystalline
lens of the eye and in front of the retina.

Coded Elsewhere:

Congenital anomalies of the vitreous (LA13.0)

9B80

Inherited vitreoretinal disorders
Coded Elsewhere:

Stickler syndrome (LD2F.1Y)

9B81

Posterior vitreous detachment

9B82

Vitreous prolapse
Exclusions:

vitreous syndrome following cataract surgery (9D20)

9B83

Vitreous haemorrhage

9B84

Vitreous opacities, membranes or strands

9B8Y

Other specified disorders of the vitreous body

9B8Z

Disorders of the vitreous body, unspecified

9C0Y

Other specified disorders of the eyeball - posterior segment

9C0Z

Disorders of the eyeball - posterior segment, unspecified

Disorders of the eyeball affecting both anterior and posterior segments

(BlockL1‑9C2)

9C20

Panuveitis

9C20.0

Noninfectious panuveitis

Coded Elsewhere:

Multifocal choroiditis (9B65.0)

9C20.1

Infectious panuveitis

Coded Elsewhere:

Tuberculous panuveitis (1B12.1)

9C20.2

Purulent endophthalmitis
Suppurative inflammation of the tissues of the internal structures of the eye; often
caused by fungi, necrosis of intraocular tumours, or retained intraocular foreign
bodies. Other aetiology can be any infectious uveitis.

9C20.Y

Other specified panuveitis

9C20.Z

Panuveitis, unspecified

9C21

Endophthalmitis
Coded Elsewhere:

Purulent endophthalmitis (9C20.2)

9C21.0

Sympathetic uveitis

32

ICD-11 MMS – 09/2020

9C21.Y

Other specified endophthalmitis

9C21.Z

Endophthalmitis, unspecified

9C22

Eyeball deformity
Coded Elsewhere:

Microphthalmos (LA10.0)

Clinical anophthalmos (LA10.1)

Microphthalmos associated with syndromes (LD21.0)

9C22.0

Atrophic Bulbi

9C22.1

Phthisis Bulbi

9C22.Y

Other specified eyeball deformity

9C22.Z

Eyeball deformity, unspecified

9C2Y

Other specified disorders of the eyeball affecting both anterior and

posterior segments

9C2Z

Disorders of the eyeball affecting both anterior and posterior segments,

unspecified

Disorders of the visual pathways or centres (BlockL1‑9C4)

This refers to disorders part of the central nervous system which gives organisms the ability to
process visual detail, as well as enabling the formation of several non-image photo response
functions.

9C40

Disorder of the optic nerve
Coded Elsewhere:

Congenital malformation of optic disc (LA13.7)

Injury of optic nerve, unilateral (NA04.10)

Malignant neoplasm of the optic nerve (2A02.12)

9C40.0

Infectious optic neuropathy

Coded Elsewhere:

Late syphilitic retrobulbar neuritis (1A62.20)

9C40.1

Optic neuritis
Optic neuritis is a condition related to immune mediated inflammation of the optic
nerve. It is commoner in women and can be the first presenting symptom of MS.
The symptoms are those of blurred vision, pain on moving the eye and in the vast
majority it is self limiting”.

Coded Elsewhere:

Neuromyelitis optica (8A43)

9C40.10

Retrobulbar neuritis

9C40.1Y

Other specified optic neuritis

9C40.1Z

Optic neuritis, unspecified

9C40.2

Neuroretinitis

INTERNATIONAL CLASSIFICATION OF DISEASES -

Mortality and Morbidity Statistics

ICD-11 MMS – 09/2020

33

9C40.3

Perineuritis of optic nerve
Inflammation of the optic nerve sheath without inflammation of the nerve itself

9C40.4

Ischaemic optic neuropathy
Optic nerve disorders caused by an ischaemic process of the optic nerve

9C40.40

Anterior ischemic optic neuropathy
This refers to anterior ischemic damage to the optic nerve due to any cause.
Damage and death of these nerve cells, or neurons, leads to characteristic features
of optic neuropathy.

9C40.41

Posterior ischemic optic neuropathy
This refers to posterior ischemic damage to the optic nerve due to any cause.
Damage and death of these nerve cells, or neurons, leads to characteristic features
of optic neuropathy.

9C40.4Y

Other specified ischaemic optic neuropathy

9C40.4Z

Ischaemic optic neuropathy, unspecified

9C40.5

Compressive optic neuropathy
Optic nerve disorders caused by the compression of the optic nerve

9C40.6

Infiltrative optic neuropathy
Optic nerve disorders caused by an infiltrative process of the optic nerve

9C40.7

Traumatic optic neuropathy
Optic nerve disorders due to trauma to the optic nerve

9C40.8

Hereditary optic neuropathy
Optic nerve disorders caused by genetic abnormalities

Coded Elsewhere:

Leber hereditary optic neuropathy (8C73.Y)

9C40.9

Glaucomatous optic neuropathy

Inclusions:

Glaucomatous optic atrophy

9C40.A

Optic disc swelling
This refers to swelling in the location where ganglion cell axons exit the eye to form
the optic nerve. There are no light sensitive rods or cones to respond to a light
stimulus at this point.

Coding Note:

Code aslo the casusing condition

9C40.A0

Papilloedema
Optic disc swelling that results from increased intracranial pressure

Inclusions:

Optic disc swelling that results from increased intracranial

pressure

9C40.A1

Optic disc swelling associated with uveitis

34

ICD-11 MMS – 09/2020

9C40.AY

Other specified optic disc swelling

Coding Note:

Code aslo the casusing condition

9C40.AZ

Optic disc swelling, unspecified

Coding Note:

Code aslo the casusing condition

9C40.B

Optic atrophy
Optic atrophies (OA) refer to a specific group of hereditary optic neuropathies in
which the cause of the optic nerve dysfunction is inherited either in an autosomal
dominant or autosomal recessive pattern. Autosomal dominant optic atrophy
(ADOA), type Kjer, is the most common OA, whereas autosomal recessive optic
atrophy (AROA) is a rare form.

Coded Elsewhere:

Leber hereditary optic neuropathy (8C73.Y)

9C40.B0

Congenital optic atrophy

9C40.B1

Acquired optic atrophy

Coding Note:

Code aslo the casusing condition

9C40.BZ

Optic atrophy, unspecified

9C40.Y

Other specified disorder of the optic nerve

9C40.Z

Disorder of the optic nerve, unspecified

9C41

Disorder of optic chiasm

This is a group of conditions associated with the optic chiasm, the part of the brain
where the optic nerves (CN II) partially cross.

Coding Note:

Use additional code, if desired, to identify underlying condition.

9C42

Disorder of post chiasmal visual pathways

Coding Note:

Use additional code, if desired, to identify underlying condition.

Inclusions:

Disorders of optic tracts, geniculate nuclei and optic radiations

9C43

Disorder of visual cortex

Coding Note:

Use additional code, if desired, to identify underlying condition.

9C44

Disorder of higher visual centres

Coding Note:

Use additional code, if desired, to identify underlying condition.

9C4Y

Other specified disorders of the visual pathways or centres

9C4Z

Disorders of the visual pathways or centres, unspecified

Glaucoma or glaucoma suspect (BlockL1‑9C6)

9C60

Glaucoma suspect

9C61

Glaucoma

INTERNATIONAL CLASSIFICATION OF DISEASES -

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35

Exclusions:

Traumatic glaucoma due to birth injury (KA41)

Coded Elsewhere:

Glaucomatous optic neuropathy (9C40.9)

9C61.0

Primary open-angle glaucoma
Primary open-angle glaucoma is a chronic progressive optic neuropathy with
characteristic morphological changes at the optic nerve head and retinal nerve fibre
layer in the absence of other ocular disease or congenital anomalies. Progressive
retinal ganglion cell death and visual field loss are associated with these changes.
Anterior chamber angle appearance is normal and major risk factors include level of
intraocular pressure and older age.

9C61.00

Normal tension glaucoma
Normal tension glaucoma is a condition considered to be within the continuum of
primary open-angle glaucoma; the term is used when intraocular pressure is within
the statistically normal range (10-21 mmHg).

9C61.01

Ocular hypertension
Ocular hypertension is a condition of elevated intraocular pressure in the absence
of optic nerve, nerve fibre layer or visual field abnormalities.

9C61.0Y

Other specified primary open-angle glaucoma

9C61.0Z

Primary open-angle glaucoma, unspecified

9C61.1

Primary angle closure and angle closure glaucoma
Primary angle closure glaucoma is a condition described as angle closure or/and
peripheral anterior synechiae with elevated intraocular pressure and evidence of
optic nerve damage.

9C61.10

Primary angle closure suspect or anatomical narrow angle
Primary angle closure glaucoma suspect is a condition of narrow anterior chamber
angle, suspicious for future closure, with no signs of trabecular meshwork or optic
nerve damage.

9C61.11

Primary angle-closure
Primary angle closure is a condition defined by the presence of iridotrabecular
contact with elevated intraocular pressure or peripheral anterior synechiae but no
signs of optic nerve damage.

9C61.12

Primary angle closure glaucoma
Primary angle closure glaucoma is a condition described as angle closure or/and
peripheral anterior synechiae with elevated intraocular pressure and evidence of
optic nerve damage.

9C61.13

Primary angle closure without pupillary block
Primary angle closure without pupillary block Is a condition described as anatomical
variation in the iris root in which narrowing of the anterior chamber angle occurs
independent of pupillary block causing angle closure.

9C61.14

Acute angle closure with pupillary block

36

ICD-11 MMS – 09/2020

Acute Angle Closure (AAC) with pupillary block is a condition described as
circumferential iris apposition to the trabecular meshwork with rapid and excessive
increase in intraocular pressure that does not resolve spontaneously.

9C61.15

Intermittent angle-closure
Intermittent Angle Closure is a milder clinical manifestation of acute angle closure
that resolves spontaneously.

9C61.16

Chronic angle-closure
Chronic angle closure is a condition when intraocular pressure elevation is due to
variable portions of anterior chamber angle being permanently closed by peripheral
anterior synechiae.

9C61.17

Condition after acute angle-closure glaucoma attack
Condition after acute angle closure glaucoma attack refers to a condition after a
previous episode of acute angle-closure attack, usually with secondary alterations
of the iris (sphincter lesions) and lens (“Glaukomflecken”, cataract).

9C61.1Y

Other specified primary angle closure and angle closure glaucoma

9C61.1Z

Primary angle closure and angle closure glaucoma, unspecified

9C61.2

Secondary open-angle glaucoma

Coding Note:

Code aslo the casusing condition

Coded Elsewhere:

Glaucoma due to ocular surgery or laser (9D25)

9C61.20

Pseudoexfoliative open-angle glaucoma
Pseudoexfoliative

Open-Angle

glaucoma

is

a

condition

where

fibrillar

pseudoexfoliative material is produced by various ocular tissues and is deposited
on the trabecular meshwork, lens, and other structures of the anterior segment
leading to intraocular pressure elevation and subsequent optic nerve damage.

9C61.21

Pigmentary open-angle glaucoma
Pigmentary Open-Angle glaucoma Is a condition where pigment is liberated due to
rubbing of the zonules against the posterior iris sheath that leads to obstruction of
the trabecular meshwork causing intraocular pressure elevation and subsequent
optic nerve damage.

9C61.22

Lens-induced secondary open-angle glaucoma

9C61.23

Glaucoma associated with intraocular haemorrhage
Ghost cell glaucoma is a condition where bleeding into the vitreous body or anterior
chamber can lead to intraocular pressure elevation when stiffer red blood cells that
have lost their haemoglobin obstruct the trabecular meshwork.

Inclusions:

ghost cell glaucoma

9C61.24

Glaucoma due to eye inflammation

Coding Note:

Code aslo the casusing condition

9C61.25

Glaucomato-cyclitic crisis

INTERNATIONAL CLASSIFICATION OF DISEASES -

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37

A glaucomato-cyclitic crisis presents with mild keratic precipitates and aqueous
flare, acute intraocular pressure elevation and optic nerve damage when repeated
attacks occur.

9C61.26

Secondary open-angle glaucoma due to parasitic eye disease

Coding Note:

Code aslo the casusing condition

9C61.27

Glaucoma due to intraocular tumours

Coding Note:

Code aslo the casusing condition

9C61.28

Glaucoma associated with retinal detachment

Coding Note:

Code aslo the casusing condition

9C61.29

Glaucoma due to eye trauma

Coding Note:

Code aslo the casusing condition

9C61.2A

Glaucoma due to drugs

9C61.2B

Glaucoma caused by increased episcleral venous pressure

Coding Note:

Code aslo the casusing condition

9C61.2C

Secondary glaucoma due to extra-ocular mass

Coding Note:

Code aslo the casusing condition

9C61.2Y

Other specified secondary open-angle glaucoma

Coding Note:

Code aslo the casusing condition

9C61.2Z

Secondary open-angle glaucoma, unspecified

Coding Note:

Code aslo the casusing condition

9C61.3

Secondary angle closure glaucoma

Coding Note:

Code aslo the casusing condition

9C61.30

Secondary angle closure glaucoma with pupillary block
Secondary angle closure glaucoma with pupillary block is a condition where an
anteriorly subluxated lens occludes the pupil causing acute secondary angle closure
and intraocular pressure elevation.

Coding Note:

Code aslo the casusing condition

9C61.31

Secondary angle closure glaucoma without pupillary block

9C61.32

Neovascular secondary angle closure glaucoma
Neovascular secondary angle-closure glaucoma is a frequent condition where
neovascular membranes occlude and close the chamber angle by fibrovascular
contraction leading to intraocular pressure elevation and subsequent optic nerve
damage. Neovascularization can be due to retinal venous occlusion, diabetic
retinopathy, ocular ischemia, long-standing retinal detachment and other ischemic
conditions of the eye.

Coding Note:

Code aslo the casusing condition

38

ICD-11 MMS – 09/2020

9C61.33

Secondary angle closure glaucoma due to endothelial overgrowth
Secondary angle-closure glaucoma due to endothelial overgrowth is a condition
where corneal endothelial cells overgrow the trabecular meshwork and iris, closing
the angle by tissue contraction leading to intraocular pressure elevation and
subsequent optic nerve damage.

9C61.34

Secondary angle closure glaucoma due to epithelial ingrowth
Epithelial ingrowth is a condition after open globe trauma or surgery where
conjunctival or corneal epithelial cells get access to the anterior chamber and
overgrow the trabecular meshwork with subsequent intraocular pressure elevation
and optic nerve damage.

9C61.35

Ciliary block glaucoma
Ciliary block glaucoma is a condition where aqueous misdirection into the vitreous
cavity displaces the lens-iris diaphragm anteriorly thus causing angle closure with
subsequent intraocular pressure elevation and optic nerve damage.

9C61.36

Secondary angle closure glaucoma due to other anterior displacement of the lens-

iris diaphragm
Iris and ciliary body cysts, intraocular tumours, posterior scleritis, uveal effusion, or
Silicon Oil or gas in the vitreous cavity can cause IOP elevation by angle closure.

9C61.3Y

Other specified secondary angle closure glaucoma

Coding Note:

Code aslo the casusing condition

9C61.3Z

Secondary angle closure glaucoma, unspecified

Coding Note:

Code aslo the casusing condition

9C61.4

Developmental glaucoma

Inclusions:

Glaucoma of newborn

Hydrophthalmos

9C61.40

Primary congenital glaucoma
Primary Congenital Glaucoma is a condition during early infancy where delayed
development and malformation of the trabecular meshwork blocks the outflow
routes leading to elevated intraocular pressure that causes enlargement of the
eyeball (Buphthalmus), corneal oedema, Descemet tears, myopia, and damage to
the optic nerve, often resulting in severe visual impairment or blindness

9C61.41

Primary infantile glaucoma
Primary infantile glaucoma is a condition after 2 years of age where malformation
of the trabecular meshwork causes elevated intraocular pressure without
enlargement of the eyeball but damage to the optic nerve similar to congenital
glaucoma.

9C61.42

Secondary childhood glaucoma

Coding Note:

Code aslo the casusing condition

Coded Elsewhere:

Aniridia (LA11.3)

INTERNATIONAL CLASSIFICATION OF DISEASES -

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39

Marfan syndrome (LD28.01)

Rubella (1F02)

Oculocerebrorenal syndrome (5C60.0)

Neurofibromatoses (LD2D.1)

9C61.4Y

Other specified developmental glaucoma

9C61.4Z

Developmental glaucoma, unspecified

9C61.Z

Glaucoma, unspecified

9C6Y

Other specified glaucoma or glaucoma suspect

9C6Z

Glaucoma or glaucoma suspect, unspecified

Strabismus or ocular motility disorders (BlockL1‑9C8)

Disorder due to abnormalities of extraocular muscles or ocular motor abnormalities.

Coded Elsewhere:

Diseases of neuromuscular junction or muscle (8C60-8D0Z)

9C80

Non paralytic strabismus

Non-paralytic strabismus is an abnormal binocular alignment in which one of the
eyes is deviated. There are full ocular movements in each eye. The condition can
alternate between eyes or only involve one eye. Strabismus may be intermittent or
constant. The abnormal alignment may be present at distance fixation, near fixation
or both.

9C80.0

Esotropia
Esotropia is an abnormal binocular alignment in which one of the eyes has an
inward deviation. Fixation can be alternatively or monocular. Esotropia is present in
all distances. Squint angles can vary with distances.

9C80.1

Exotropia
Exotropia is an abnormal binocular alignment in which one of the eyes has an
outward deviation. Fixation can be alternatively or monocular. Exotropia is present
in all distances.

9C80.2

Vertical or torsional strabismus
An abnormal binocular alignment which may be constant or intermittent, that is not
horizontal, but vertical or torsional (rotational) around the pupillary axis.

9C80.3

Intermittent strabismus
An abnormal binocular alignment which is present intermittently, with normal
alignment at other times with binocular single vision.

9C80.30

Intermittent divergent exotropia

9C80.31

Intermittent convergent esotropia

9C80.3Y

Other specified intermittent strabismus

40

ICD-11 MMS – 09/2020

9C80.3Z

Intermittent strabismus, unspecified

9C80.4

Heterophoria
A temporary deviation of the eyes from normal binocular alignment when there is
disruption of the visual input from one eye. The alignment is normal when there is
binocular visual input.

9C80.5

Mechanical strabismus
An abnormal binocular alignment caused by abnormalities of ocular movement in
one or both eyes caused by damage to the extraocular muscles and/or other orbital
structures. Mechanical strabismus is characterised by limitation of movements in
one or more directions and variable strabismus.

9C80.Y

Other specified non paralytic strabismus

9C80.Z

Non paralytic strabismus, unspecified

9C81

Ocular motor nerve palsies
Exclusions:

Internuclear ophthalmoplegia (9C83.5)

Internal ophthalmoplegia (9D01.0)

ophthalmoplegia progressive supranuclear (8A00.10)

9C81.0

Third nerve palsy

Inclusions:

isolated oculomotor nerve palsy

9C81.00

External bilateral paralysis of oculomotor nerve

9C81.0Y

Other specified third nerve palsy

9C81.0Z

Third nerve palsy, unspecified

9C81.1

Fourth nerve palsy

Inclusions:

isolated trochlear nerve palsy

9C81.2

Sixth nerve palsy

Inclusions:

isolated abducent nerve palsy

9C81.3

Total external ophthalmoplegia

9C81.4

Cavernous sinus syndromes

9C81.Y

Other specified ocular motor nerve palsies

9C81.Z

Ocular motor nerve palsies, unspecified

9C82

Disorders of extraocular muscles
Coded Elsewhere:

Certain paralytic strabismus (9C81.Y)

9C82.0

Progressive external ophthalmoplegia
Chronic ophthalmoplegia is characterised by progressive weakness of ocular
muscles and levator muscle of the upper eyelid. The condition is mainly manifested
in adults. It may be totally and permanently isolated, however in a minority of cases

INTERNATIONAL CLASSIFICATION OF DISEASES -

Mortality and Morbidity Statistics

ICD-11 MMS – 09/2020

41

it is associated with skeletal myopathy, which causes abnormal fatigability and
even permanent muscle weakness. In this case the affection is still termed isolated
progressive

external

ophthalmoplegia.

A

large

proportion

of

chronic

ophthalmoplegias presents with multisystemic pattern of signs: neurological signs
(hearing loss, retinopathy, cerebellar disorders, peripheral neuropathy, etc.),
endocrine (diabetes, hypogonadism, hypoparathyroidism, etc.), kidney (kidney
failure,

tubulopathy,

etc.),

and

heart

disorders

(conduction

disorders,

myocardiopathy, etc.).

9C82.1

Muscular dystrophy affecting extraocular muscle
Non-specific term that is used to describe a range of primary myopathies that affect
the extraocular muscles.

Exclusions:

Secondary myopathies (BlockL2‑8C8)

Coded Elsewhere:

Congenital fibrosis of extraocular muscles (9C82.2)

9C82.2

Congenital cranial dysinnervation syndrome

9C82.3

Restrictive ophthalmopathy

Coding Note:

Code aslo the casusing condition

9C82.4

Oculomotor apraxia

9C82.Y

Other specified disorders of extraocular muscles

9C82.Z

Disorders of extraocular muscles, unspecified

9C83

Disorders of binocular movement

Other disorders of binocular movement in which the movement of the two eyes is
abnormal.

9C83.0

Palsy of conjugate gaze
A palsy of conjugate gaze is an incomplete or absent movement of the two eyes in
a particular direction of gaze.

Coded Elsewhere:

Progressive supranuclear palsy (8A00.10)

9C83.00

Horizontal gaze palsy
A palsy of horizontal gaze is an incomplete or absent movement of the two eyes in
a horizontal direction of gaze. May be in one or both directions

9C83.01

Vertical gaze palsy
A palsy of vertical gaze is an incomplete or absent movement of the two eyes in the
vertical direction of gaze.

9C83.02

Monocular elevator palsy
Monocular elevator palsy is an incomplete or absent movement of one eyes in
upgaze. May be due to pathology in the orbit, as well as infranuclear, internuclear, or
supranuclear in origin.

9C83.0Y

Other specified palsy of conjugate gaze

9C83.0Z

Palsy of conjugate gaze, unspecified

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ICD-11 MMS – 09/2020

9C83.1

Spasm of conjugate gaze

9C83.10

Horizontal conjugate gaze deviation

9C83.11

Upward gaze deviation

9C83.12

Downward gaze deviation

9C83.13

Oculogyric crisis
Episodic spells of tonic upward and sometimes laterally deviation of the eyes, rarely
downward

9C83.1Y

Other specified spasm of conjugate gaze

9C83.1Z

Spasm of conjugate gaze, unspecified

9C83.2

Convergence insufficiency

9C83.3

Convergence excess

9C83.4

Spasm of the near reflex

9C83.5

Internuclear ophthalmoplegia
This is a disorder of conjugate lateral gaze in which the affected eye shows
impairment of adduction.

9C83.6

Anomalies of divergence or deviation of eye movement

9C83.60

Divergence insufficiency

9C83.61

Divergence paralysis

9C83.62

Divergence excess

9C83.63

Synergistic divergence
Anomalous innervation of muscle normally supplied by the oculomotor nerve.

In congenital unilateral adduction palsy, when adduction is attempted, the affected
eye abducts rather than adducts.

9C83.64

Skew deviation
a vertical misalignment of the visual axes caused by a disturbance of prenuclear
inputs

9C83.65

Ocular tilt reaction
skew deviation associated with ocular torsion (cyclodeviation) and a head tilt (ear to
shoulder)

9C83.66

Alternating skew deviation

9C83.67

Dissociative vertical divergence

9C83.6Y

Other specified anomalies of divergence or deviation of eye movement

9C83.6Z

Anomalies of divergence or deviation of eye movement, unspecified

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ICD-11 MMS – 09/2020

43

9C83.Y

Other specified disorders of binocular movement

9C83.Z

Disorders of binocular movement, unspecified

9C84

Nystagmus

9C84.0

Physiological nystagmus

9C84.1

Congenital forms of nystagmus

9C84.2

Vestibular nystagmus

9C84.20

Down beat nystagmus

9C84.21

Upbeat nystagmus

9C84.22

Torsional nystagmus

9C84.23

Perverted nystagmus

9C84.2Y

Other specified vestibular nystagmus

9C84.2Z

Vestibular nystagmus, unspecified

9C84.3

Seesaw nystagmus

9C84.4

Gaze-evoked nystagmus

9C84.5

Nystagmus occurring in visual system disorders

Coding Note:

Code aslo the casusing condition

Coded Elsewhere:

Spasmus nutans (8A04.Y)

9C84.50

Visual deprivation nystagmus

9C84.51

Divergence nystagmus

9C84.52

Convergence-retraction nystagmus

9C84.5Y

Other specified nystagmus occurring in visual system disorders

Coding Note:

Code aslo the casusing condition

9C84.5Z

Nystagmus occurring in visual system disorders, unspecified

Coding Note:

Code aslo the casusing condition

9C84.6

Eyelid nystagmus

9C84.Y

Other specified nystagmus

9C84.Z

Nystagmus, unspecified

9C85

Certain specified irregular eye movements

9C85.0

Anomalies of saccadic eye movements

9C85.00

Disorders of the saccadic pulse

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ICD-11 MMS – 09/2020

9C85.01

Disorders of the saccadic step

Coded Elsewhere:

Gaze-evoked nystagmus (9C84.4)

9C85.02

Inappropriate saccades

Inclusions:

Saccadic intrusions and oscillations

9C85.0Y

Other specified anomalies of saccadic eye movements

9C85.0Z

Anomalies of saccadic eye movements, unspecified

9C85.1

Anomalies of smooth pursuit movements

9C85.2

Nonorganic eye movement disorders

9C85.Y

Other specified irregular eye movements

9C85.Z

Irregular eye movements, unspecified

9C8Y

Other specified strabismus or ocular motility disorders

9C8Z

Strabismus or ocular motility disorders, unspecified

Disorders of refraction or accommodation (BlockL1‑9D0)

9D00

Disorders of refraction

9D00.0

Myopia
A refractive error in which rays of light entering the eye parallel to the optic axis are
brought to a focus in front of the retina when ocular accommodation is relaxed.
This usually results from the eyeball being too long from front to back, but can be
caused by an overly curved cornea, a lens with increased optical power, or both. It is
also called nearsightedness.

Exclusions:

degenerative myopia (9B76)

9D00.1

Hypermetropia
A refractive error in which rays of light entering the eye parallel to the optic axis are
brought to a focus behind the retina, as a result of the eyeball being too short from
front to back. It is also called farsightedness because the near point is more distant
than it is in emmetropia with an equal amplitude of accommodation.

9D00.2

Astigmatism
Unequal curvature of the refractive surfaces of the eye. Thus a point source of light
cannot be brought to a point focus on the retina but is spread over a more or less
diffuse area. This results from the radius of curvature in one plane being longer or
shorter than the radius at right angles to it.

9D00.3

Presbyopia
The normal decreasing elasticity of the crystalline lens that leads to loss of
accommodation.

9D00.4

Anisometropia

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ICD-11 MMS – 09/2020

45

9D00.5

Aniseikonia

9D00.6

Transient refractive change

9D00.Y

Other specified disorders of refraction

9D00.Z

Disorders of refraction, unspecified

9D01

Disorders of accommodation

9D01.0

Internal ophthalmoplegia

9D01.1

Paresis of accommodation

9D01.2

Spasm of accommodation

9D01.Y

Other specified disorders of accommodation

9D01.Z

Disorders of accommodation, unspecified

9D0Y

Other specified disorders of refraction or accommodation

9D0Z

Disorders of refraction or accommodation, unspecified

Postprocedural disorders of eye or ocular adnexa (BlockL1‑9D2)

Exclusions:

pseudophakia (QB51.2)

Coded Elsewhere:

Haemorrhage and haematoma of eye or ocular adnexa complicating a procedure

(NE81.01)

Injury or harm arising from surgical or medical care, not elsewhere classified

(NE80-NE8Z)

9D20

Bullous aphakic keratopathy following cataract surgery
Inclusions:

Vitreal corneal syndrome

9D21

Cataract lens fragments in eye following cataract surgery

9D22

Chorioretinal scars after surgery for detachment

9D23

Conjunctival blebitis after glaucoma surgery

9D24

Complications with glaucoma drainage devices

9D25

Glaucoma due to ocular surgery or laser

Impairment of visual functions (BlockL1‑9D4)

Coded Elsewhere:

Impairment of electrophysiological functions (MC21)

Polyopia (9D53)

9D40

Impairment of visual acuity

Visual acuity refers to the ability to recognize details at the point of fixation, which
usually is the fovea. It is expressed as an angular measure, usually measured as

46

ICD-11 MMS – 09/2020

distance and/or near acuity.

9D41

Impairment of visual field

Ranges of visual field impairment refer to the extent of peripheral vision outside
fixation. The extent should be measured for each eye separately.

9D42

Patterns of visual field impairment

Patterns of visual field impairment are often indicative for certain disease
conditions.

9D42.0

Visual field loss, pattern not specified

9D42.1

Normal Visual Field

9D42.2

Peripheral field deficit

9D42.20

Enlarged blind spot

Inclusions:

Scotoma of blind spot area

9D42.21

Arcuate scotoma
A Bjerrum or arcuate scotoma follows the pattern of the retinal nerve fibres. It is
typical for glaucomatous defects and can also be caused by juxta-papillary lesions.

9D42.22

Nasal step
A nasal step is a discontinuity of the nasal field limit at the horizontal meridian. It is
typical for glaucoma.

9D42.23

Ring scotoma
A ring scotoma is a scotoma that surrounds the central field. Initially, it may consist
of several smaller scotomas that gradually coalesce.

9D42.24

Isolated peripheral scotoma
Isolated scotomas may be the result of scarring from infections or surgery.

9D42.2Y

Other specified peripheral field deficit

9D42.2Z

Peripheral field deficit, unspecified

9D42.3

Hemianopic or quadrantic loss
Defects that cover a hemi-field or a quadrant in one eye may be the result of optic
nerve involvement.

9D42.4

Central scotoma
A central scotoma is a defect that covers the fovea. It therefore causes visual acuity
loss and may necessitate eccentric fixation.

9D42.5

Para-central scotoma
A para-central scotoma is a scotoma adjacent to the fovea. Both may minimally
affect letter chart acuity, but may interfere significantly with reading and other
activities.

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47

9D42.6

Homonymous hemianopia or quadrant anopia
Homonymous, binocular field defects present the same or similar patterns in both
eyes. They are caused by lesions of the retro-chiasmal pathways.

9D42.60

Right hemi-field homonymous hemianopia or quadrant anopia

9D42.61

Left hemi-field homonymous hemianopia or quadrant anopia

9D42.6Y

Other specified homonymous hemianopia or quadrant anopia

9D42.6Z

Homonymous hemianopia or quadrant anopia, unspecified

9D42.7

Heteronymous hemianopia or quadrant anopia
Heteronymous field defects present opposite patterns in the two eyes. They are
caused by may be caused by-chiasmal lesions.

9D42.70

Bi-nasal defects heteronymous hemianopia or quadrant anopia

9D42.71

Bi-temporal defects heteronymous hemianopia or quadrant anopia

9D42.7Y

Other specified heteronymous hemianopia or quadrant anopia

9D42.7Z

Heteronymous hemianopia or quadrant anopia, unspecified

9D42.8

Visual field loss, other specified forms

9D42.Y

Other specified patterns of visual field impairment

9D42.Z

Patterns of visual field impairment, unspecified

9D43

Impairment of contrast vision

Contrast sensitivity refers to the ability to distinguish small differences in
brightness between adjacent surfaces.

Peak Contrast sensitivity refers to the smallest differences that are discernible for
large stimuli.

For smaller objects, such as those involved in many Activities of Daily Living,
contrast sensitivity interacts with visual acuity and visual field. Better contrast
makes smaller details visible. The visual field is larger for stronger stimuli.

9D44

Impairment of colour vision

Colour vision refers to the ability to distinguish colour differences. True colour
“blindness” is extremely rare. Most colour vision deficiencies are minor, and
congenital, with X-linked recessive inheritance (more prevalent among men). Some
drugs and optic neuritis may also cause colour vision deficiencies.

Inclusions:

achromatopsia

acquired colour vision deficiency

colour blindness

9D45

Impairment of light sensitivity
Coded Elsewhere:

Vitamin A deficiency with night blindness (5B55.0)

9D46

Impairment of binocular functions

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ICD-11 MMS – 09/2020

Subjective visual experiences (BlockL2‑9D5)

Subjective Visual Experiences are experiences reported by patients, whose presence or absence
cannot be verified objectively.

9D50

Visual discomfort
Inclusions:

Asthenopia

9D51

Transient visual loss
Coded Elsewhere:

Amaurosis fugax (8B10.0)

9D52

Hemifield losses

9D53

Entoptic phenomena

Entoptic phenomena are visual phenomena caused by changes within the eye.

Coded Elsewhere:

Visual floaters (MC1A)

9D54

Visual illusions

Visual illusions refer to percepts based on an erroneous interpretation of visual
input.

9D55

Nonorganic visual loss

9D56

Visual release hallucinations

Charles Bonnet syndrome, also called visual release hallucinations, refers to the
experience of complex visual hallucinations in a person who has experienced partial
or complete loss of vision. Hallucinations are exclusively visual, usually temporary,
and unrelated to mental and behavioural disorders.

Exclusions:

Schizophrenia or other primary psychotic disorders

(BlockL1‑6A2)

9D5Y

Other specified subjective visual experiences

9D5Z

Subjective visual experiences, unspecified

9D7Y

Other specified impairment of visual functions

9D7Z

Impairment of visual functions, unspecified

Vision impairment (BlockL1‑9D9)

Visual Disability refers to deficits in the ability of the person to perform vision-related activities of
daily living, such as: reading, orientation and mobility, and other tasks.

Visual disability scores reflect the Burden of Vision Loss for the person, and should be assessed with
both eyes open and with presenting correction (if any).

9D90

Vision impairment including blindness

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49

9D90.0

No vision impairment

9D90.1

Mild vision impairment

9D90.2

Moderate vision impairment

Inclusions:

visual impairment categories 1 or 2 in both eyes

Visual impairment category 1

WHO - low vision

9D90.3

Severe vision impairment

Inclusions:

Legal blindness - USA

visual impairment categories 3, 4, 5 in one eye, with categories

1 or 2 in the other eye

9D90.4

Blindness, binocular

Inclusions:

visual impairment categories 3, 4, 5 in both eyes.

Visual impairment category 5

9D90.5

Blindness, monocular

Inclusions:

visual impairment categories 3, 4, 5 in one eye [normal vision

in other eye].

Visual impairment categories 3, 4, 5 in one eye and categories

0, 1, 2 or 9 in the other eye.

9D90.Y

Other specified vision impairment including blindness

9D90.Z

Vision impairment including blindness, unspecified

9D91

Near vision deficits

Near vision refers to the ability to perform tasks that require detailed vision at a
close distance. It should be measured with both eyes open at the subject’s
preferred viewing distance and with the subject’s habitual near vision correction (if
any). Near vision impairment is characterised by a presenting near visual acuity
worse than N6.

9D92

Specific vision dysfunctions

Specific visual dysfunctions refer to functional deficits in higher cerebral centres.
Such dysfunctions may exist with or without visual impairment of the eyes and the
lower visual system.

9D93

Complex vision-related dysfunctions

Complex Vision-Related Dysfunctions involve interactions with other sensory and
motor systems. They reflect the combined effects at all stages of processing.

9D9Y

Other specified vision impairment

9D9Z

Vision impairment, unspecified

9E1Y

Other specified diseases of the visual system

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ICD-11 MMS – 09/2020

9E1Z

Diseases of the visual system, unspecified